title: Should we always plan a Fontan completion after a Kawashima procedure?
abstract: OBJECTIVE
  To determine the late incidence of pulmonary arteriovenous malformations after bidirectional Glenn in patients with azygos continuation of the inferior vena cava (Kawashima operation).
  
  
  METHODS
  From 1990 to 2006, 21 patients underwent a Kawashima procedure at a median age of 2.3 years (0.5-8 years). Underlying anatomy included atrioventricular septal defect [14], double-outlet right ventricle [13], pulmonary atresia [3], pulmonary stenosis [11], total anomalous pulmonary venous drainage [4] and bilateral superior vena cavae [14].
  
  
  RESULTS
  There was one hospital death after Kawashima due to low output syndrome. Follow-up was complete in all but one patient. Two patients died 23 days and 4 years after Kawashima following cardiac reinterventions (one atrioventricular valve replacement and one Fontan completion). Thirteen patients developed pulmonary arteriovenous malformations after a median of 4 years (2-9 years) after Kawashima. Freedom from development of arteriovenous malformations was 47% at 5 years (95% CI: 23-69%). A total of 16 patients underwent Fontan completion, 12 for cyanosis related to pulmonary arteriovenous malformations and four for decreased exercise capacity. Only three patients were left without Fontan completion at 4, 9 and 13 years after Kawashima. The two patients who had more than 9 years of follow-up after Kawashima had antegrade flow preserved between the ventricle and the pulmonary arteries.
  
  
  CONCLUSIONS
  Unless some hepatic blood flow is directed to both lungs, most, if not all patients with a Kawashima procedure will ultimately develop pulmonary arteriovenous malformations. Elective non-fenestrated Fontan completion in the years following Kawashima procedure should be recommended.
authors.affiliation: Department of Cardiac Surgery, Royal Children's Hospital, Murdoch Children's Research Institute, Melbourne, VIC, Australia.
authors.forename: Stella
authors.initials: S
authors.lastname: Setyapranata
authors.forename: Christian P
authors.initials: CP
authors.lastname: Brizard
authors.forename: Igor E
authors.initials: IE
authors.lastname: Konstantinov
authors.forename: Ajay
authors.initials: A
authors.lastname: Iyengar
authors.forename: Michael
authors.initials: M
authors.lastname: Cheung
authors.forename: Yves
authors.initials: Y
authors.lastname: d'Udekem
mesh_terms: D001165:Arteriovenous Malformations / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D004812:Epidemiologic Methods; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects* / Q000379:methods; D006330:Heart Defects, Congenital / Q000601:surgery; D006801:Humans; D007223:Infant; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000000981:diagnostic imaging; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000000981:diagnostic imaging; D011859:Radiography; D016896:Treatment Outcome; D014682:Vena Cava, Inferior / Q000002:abnormalities / Q000601:surgery